The Family That Couldn't Sleep: A Medical Mystery (Paperback)

by D.T. Max (Author)
Key Phrases: prion researchers, scrapie researcher, kuru victims, Creutzfeldt Jakob, New Guinea, Sir Mac (more...)

Editorial Reviews

From Publishers Weekly
In 1765, Venetian doctors were stumped by the death of a man who had suffered from insomnia for more than a year and spent his final months paralyzed by exhaustion. Over the next two centuries, many of his descendants would develop similarly fatal symptoms, with a range of misdiagnoses, from encephalitis to alcohol withdrawal. Finally, in the early 1990s, their disease was recognized as a rare genetic form of prion disease. The family reluctantly shared their history with Max, who has written about science and literature for the New York Times Magazine and other publications. Max (inspired in part by his own neuromuscular disorder) has crafted a powerfully empathetic account of their efforts to make sense of their suffering and find a cure. But this is only half the story. Looking at prion disease in general, Max doubles back to the English mad-cow epidemic of the 1990s, retracing established backstories among New Guinea aboriginals and European sheep herds. There's enough fascinating material—in particular, a theory suggesting that early humans were nearly wiped out by a plague spread by cannibalism—to keep readers engaged, but they're likely to want still more about the genuinely captivating family drama. (Oct. 3)
Copyright © Reed Business Information, a division of Reed Elsevier Inc. All rights reserved. --This text refers to an out of print or unavailable edition of this title.

From The New Yorker
Beginning with the story of an Italian clan whose members die of a mysterious inability to sleep, Max traces science's tortuous path toward understanding prion diseases—a category that includes scrapie in sheep, B.S.E. in cows, and kuru, a disease spread by cannibalism which decimated one New Guinean tribe. Victims of fatal familial insomnia lose control of neuromuscular function, existing in a merciless limbo between sleep and wakefulness until they die of exhaustion. For a half century, prion diseases have baffled scientists, because the transmission of illness by proteins, which are non-living, was considered impossible. Max, who suffers from a distantly related neuromuscular disease, narrates recent advances in prion science with engaging clarity. But, as he reflects ruefully, "the neurologist can diagnose you but he can't cure you."
Copyright © 2006 Click here to subscribe to The New Yorker --This text refers to an out of print or unavailable edition of this title.

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Product Details

• Paperback: 336 pages
• Publisher: Random House Trade Paperbacks (September 11, 2007)
• Language: English
• ISBN-10: 081297252X
• ISBN-13: 978-0812972528
• Product Dimensions: 7.8 x 5.1 x 0.8 inches
• Shipping Weight: 0.8 ounces (View shipping rates and policies)
• Average Customer Review: 4.8 out of 5 stars See all reviews (26 customer reviews)
• Amazon.com Sales Rank: #69,751 in Books (See Bestsellers in Books)

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Customer Reviews

Most Helpful Customer Reviews

42 of 43 people found the following review helpful:

5.0 out of 5 stars The Scourge of Prions, December 27, 2006

By	R. Hardy "Rob Hardy" (Columbus, Mississippi USA) - See all my reviews (TOP 50 REVIEWER)    (REAL NAME)

This review is from: The Family That Couldn't Sleep: A Medical Mystery (Hardcover)

In 1765 a doctor in Venice died of what was labeled "an organic defect of the heart's sack", but he many have been the first recorded victim of a strange disorder passed down to his many descendants into the twenty-first century. It had so many weird symptoms and was so rare that the victims were frequently misdiagnosed, often being dismissed as alcoholics in withdrawal, or as having meningitis, depression, encephalitis, and many other incorrect labels. The symptoms are appalling. The illness strikes adults who have no previous significant medical problems and may have started families of their own. A victim begins to hold up the head stiffly, and then sweats profusely; family members are terrified when these initial symptoms appear, as the others follow inexorably. The pupils contract to pinpoints, the heart goes mad with increased pulse and blood pressure, and sleep becomes impossible, no matter what drugs are used to bring it on. The victim knows what is happening until dementia takes over, followed by a coma and then death in about a year or two after the symptoms began. Nothing at all can be done to stop the progress of the illness, which is passed to one half of each succeeding generation. It is, however, becoming more comprehensible as we learn more about prions, those bad proteins. In _The Family That Couldn't Sleep: A Medical Mystery_ (Random House), D. T. Max has not only told the story of this particular illness, but also of other illnesses that are (or might be) caused by prions. It is a tale full of undeserving victims and flawed heroes, and it tells just how far we are from solving some basic biological riddles.

Proteins are what DNA codes for; because prions aren't alive, they cannot be killed; radiation, formaldehyde, and all ordinary sterilization procedures do nothing to them. You might get prions by having your DNA code for them; that's what happens in the Italian family that has Fatal Family Insomnia (FFI). That's pretty rare, but you might also get prions by eating them, as in eating cows with Mad Cow Disease. There are prion diseases of sheep and deer as well. A strange neurological disease in New Guinea called kuru unlocked some of the mysteries of prions (in this case, passed by cannibalism), and Nobel prizes have stemmed from this work. One of the frustrating parts of this story is that prion afflictions have often been brought about by people. No one intended to get any animal or human sick, but human intervention made it happen. Scrapie started afflicting sheep two centuries ago as a direct result of intensive breeding to make bigger animals. Mad Cow Disease was caused by the unnatural feeding of sheep cadaver protein to cows. Chronic Wasting Disease in deer seems to have been passed to them when sheep were held in pens used for sheep with scrapie, but also may come about when deer farms, pressed to produce bucks with bigger racks for hunters, fed the deer the same sort of sheep cadaver protein that the cows got. It also got spread when humans transported these deer to different regions of the country, a migration they could not have done on their own. We attempt to control nature, and in response, nature presents us with problems no one could have foreseen.

Max has given a clear history of prion diseases and our attempts to understand them. A surprising part of the prion story is that it gives evidence that our hominid forebears practiced cannibalism, and therefore the genes of most people show a protective trait that helps keep prions from causing disease. The positive part of the story is that we are not as ignorant about prions as we were four decades ago, and even that prion research may open up answers to possibly related neuromuscular and neurodegenerative diseases like Parkinson's and Alzheimer's. But what we know is overbalanced by what we don't. Max writes, "Not since Pasteur's time have researchers attempted to counter an infection knowing so little about what they are fighting." Not only that, but prion diseases are all brand new diseases, as diseases go, and some have been manufactured in the past few decades; there is no telling what completely new one will be in tomorrow's headlines.

26 of 27 people found the following review helpful:

5.0 out of 5 stars A Real-life Mystery with All the Elements of a Fictional Blockbuster, October 8, 2006

By	Phyllis Staff (Dallas, Texas USA) - See all my reviews (TOP 500 REVIEWER)    (REAL NAME)

This review is from: The Family That Couldn't Sleep: A Medical Mystery (Hardcover)

Spanning two centuries, this book traces the origins of prions (and the terrible diseases they cause) to our current state of understanding.

The author's treatment makes this story stand out. What might have been a dry recital of discovery becomes of tale of greed, discovery, ego, opportunities both missed and taken, and the rigidity of belief. Along the way, we meet a family cursed with a genetic heritage that destroys lives with a disease that leaves the sufferer unable to sleep and fully conscious of a horrible fate.

I was particularly interested in this book because prion disease in humans is sometimes misdiagnosed as Alzheimer's. I came away with a clearer understanding of the types of prion disease and how they differ from each other and from Alzheimer's. I only wish the book could have ended with a clear answer to prevention and cure, but perhaps when that comes, Max will favor us with another tale.

Highly recommended!

Phyllis Staff, Ph.D.
author:
"How to Find Great Senior Housing," and
"128 Ways to Prevent Alzheimer's and Other Dementias"

21 of 22 people found the following review helpful:

5.0 out of 5 stars The Family That Couldn't Sleep, October 26, 2006

By	S. Dutttenhaver "S Duttenhaver" (Savannah GA) - See all my reviews (REAL NAME)

This review is from: The Family That Couldn't Sleep: A Medical Mystery (Hardcover)

This book was so fascinating, I was compelled to email the author and congratulate him on his work. The Family That Couldn't Sleep tells the thought provoking story of an emerging medical mystery that is likely to affect all of us in our lifetime. Woven into the story are fascinating details regarding the history, politics and evolving research of a potential health related epidemic in our country. Max is able to communicate a complex disease phenomena in a gripping fashion that is also accessible to lay people such as myself...medical degree not required!